SciELO - Scientific Electronic Library Online

vol.25 issue1Peritoneal pseudomyxoma: cytoreductive operation and intraperitoneal chemo-hyperthermia - Report of two casesSome considerations on "synthesis of large wounds of the body wall with rubber elastic band" author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand




Related links


ABCD. Arquivos Brasileiros de Cirurgia Digestiva (São Paulo)

Print version ISSN 0102-6720

ABCD, arq. bras. cir. dig. vol.25 no.1 São Paulo Jan./Mar. 2012 



Mirizzi syndrome in association with serum CA 19-9 greater than 20.000U/ml: is it possible?



Paulo Roberto Ott Fontes; Uirá Fernandes Teixeira; Fábio Luiz Waechter; José Artur Sampaio; Luiz Pereira-Lima

Department of Digestive Tract Surgery, Federal University of Health Sciences of Porto Alegre, UFCSPA, Santa Casa de Porto Alegre, Porto Alegre, Brazil





The CA 19-9 it a carbohydrate antigen of cellular surface described initially as a monoclonal antibody against cells of carcinoma colorectal lineage2. As tumor marker it is measured in patients with malignant diseases of pancreas and biliary tree. The elevation this glycoprotein, however, is not restricted to malignant diseases. Values higher the 1000 U / ml are exception in benign diseases4.

The present case has the goal to analysis the diagnosis of neoplasia in icteric patient with high levels of the marker.



Woman of 83 years was assisted with history of progressive jaundice, dark urine, hipocholia, pruritus, pain in right hypochondrium and weight loss. On physical examination the patient was icteric with discrete pain to palpation of right hypochondrium without evidence of palpable masses.

The laboratory tests in admission were: total bilirubin = 26, direct bilirubin = 15.8, gammaglutamyl transferase = 829, alkaline phosphatase = 518, glutamic oxaloacetic transaminase = 115, alanine aminotransferase transaminase = 92, hemoglobin = 12.7, prothrombin time = 89%, antigen carbohydrate CA19-9 = 24 480 U / mL.

Computed tomography showed important dilatation of intra and extrahepatic biliary tree (Figure 1). Was also observed apparent compression of common bile duct by gallbladder, moderately distended with thickened walls, and image of calculus in its infundibulum of 2.0 cm. The possibility of biliary neoplasia was not removed.



The patient was submitted to exploratory laparotomy in which was evidenced intense inflammatory process on triangle of Calot, with presence of fistula between the gallblader and the common bile duct. Started by opening of gallbladder was identified calculus of 2.3 cm on infundibulum, the only one found. Choledochostomy with Kehr drain and control cholangiography was done with passage of contrast to the duodenum and absence of injuries or residual calculations. The liver biopsy revealed standard biliary portal reaction and obstruction of large ducts. Other biopsies did not reveal malignancy, and were compatible with acute and chronicle inflammation and edema, confirming the diagnosis of syndrome of Mirizzi.

The patient evolved well post-operatively. Cholangiography on 9th day was normal. There was normalization of markers of cholestasis and significant decrease of serum level of CA 19-9 to 355.9 U / ml. On follow-up of three years the patient was in good state general without evidence of malignant disease and with serum levels of CA 19-9 in normality.



The syndrome of Mirizzi was described first in 1948. Was divided in four types depending of degree of compression of hepatic duct or presence of fistula. This condition can be suggested image, but many patients gave the diagnosis only at the operation with higher risk of iatrogenic lesion.

The elevation of CA 19-9 in benign diseases is reported in literature, both in biliopancreatic diseases as in other sites of gastrointestinal system 2.4. Expressive levels, however, are uncommon. The syndrome of Mirizzi appears as one of these rarities. Till nowadays, only four cases are reported and presenting CA 19-9 > 20,000 U / ml1,3,4,5.

The hyperbilirubinemia secondary to obstruction is present in majority of benign cases with elevation of CA 19-9, and the values tend the decrease or normalize after the resolution of cholestasis 2.4. Such fact was verified here.

The CA 19-9 is produced by the biliary epithelium and secreted in bile. Therefore, in cases of obstruction decrease its excretion and increase the absorption. Therefore, after the relief of jaundice, the levels tend to normalize. Such fact is rare in malignant diseases due to the production of antigen by the neoplasia2.

High levels of CA19-9 do not indicate the presence of malignancy. The syndrome of Mirizzi appears in terms to be remembered on differential diagnosis. The diagnosis of malignancy should be the first one the be sought in cases of cholestatic syndrome and high CA 19-9.



1. Lin CL, Changchien CS, Chen YS. Mirizzi's syndrome with a high CA19-9 level mimicking cholangiocarcinoma. Am J Gastroenterol. 1997;92(12):2309-10.         [ Links ]

2. Mann DV, Edwards R, Ho S, Lau WY, Glazer G. Elevated tumour marker CA19-9: clinical interpretation and influence of obstructive jaundice. Eur J Surg Oncol. 2000;26(5):474-79.         [ Links ]

3. Principe A, Del Gaudio M, Grazi GL, Paolucci U, Cavallari A. Mirizzi syndrome with cholecysto-choledocal fistula with a high CA19-9 level mimicking biliary malignancies: a case report. Hepato-gastroenterol 2003;50(53): 1259-62.         [ Links ]

4. Robertson AG, Davidson BR. Mirizzi syndrome complicating an anomalous biliary tract: a novel cause of a hugely elevated CA19-9. Eur J Gastroenterol Hepatol. 2007;19(2):167-69.         [ Links ]

5. Sanchez M, Gomes H, Marcus EN. Elevated CA 19-9 levels in a patient with Mirizzi syndrome: case report. South Med J. 2006;99(2):160-63.         [ Links ]



Paulo Roberto Ott Fontes

Financial source: none
Conflicts of interest: none



From the Department of Digestive Tract Surgery, Federal University of Health Sciences of Porto Alegre, UFCSPA, Santa Casa de Porto Alegre, Porto Alegre, Brazil.

Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License